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Noninvasive cardiac evaluation of young patient with cystic fibrosis
O Küçükosmanoğlu1, N Ozbarlas, A Göçmen
1Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara.
Insights
Early cardiac changes, including diastolic dysfunction and increased right ventricular wall thickness, are present in young cystic fibrosis patients. These findings suggest potential heart issues begin in early childhood.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Echocardiography
Background:
- Cystic fibrosis (CF) is a genetic disorder primarily affecting the lungs.
- Cardiac complications in CF are increasingly recognized, particularly in adults.
- Early cardiac assessment in pediatric CF populations is crucial for understanding disease progression.
Purpose of the Study:
- To evaluate cardiac function in young cystic fibrosis patients using echocardiography.
- To compare cardiac parameters between CF patients and a healthy control group.
- To identify early signs of cardiac involvement in pediatric CF.
Main Methods:
- Echocardiographic assessment of 26 young cystic fibrosis patients (mean age 48.4 months) and 26 age/sex-matched controls.
- Measurement of left and right ventricular dimensions, systolic function (ejection fraction, fractional shortening), and diastolic function parameters.
- Analysis of septal motion and valve velocity patterns.
Main Results:
- Patients showed normal left ventricular ejection fraction and fractional shortening but increased right ventricular free wall thickness.
- Abnormal septal motion was observed in 6 patients; diastolic function was impaired (lower E/A ratios, higher late atrial peak velocity).
- Negative correlation found between right ventricular pre-ejection period/ventricular ejection time ratio and Shwachman scores.
Conclusions:
- Cardiac changes, particularly in diastolic function and right ventricular structure, are detectable in very young cystic fibrosis patients.
- These findings indicate that cardiac involvement in CF can begin in early childhood.
- Echocardiography is valuable for detecting subclinical cardiac alterations in pediatric CF.
Abstract:
Cardiac involvement was evaluated by echocardiography in 26 young cystic fibrosis patients. The mean age was 48.4 months (range 3 months to 15 years). The findings were compared with 26 age- and sex-matched children without a history of cardiopulmonary complaints. All patients had normal values of left ventricular ejection fraction and fractional shortening. Interventricular septal and posterior left ventricular wall thicknesses were similar to control group but right ventricular free wall thickness was found greater than in the control group. Abnormal septal motion was documented in six patients. Right ventricular pre-ejection period to ventricular ejection time ratio was found over the upper limit of normal in two patients and there was a negative correlation with clinical Shwachman scores (r: -0.55). Left ventricular pre-ejection period to ventricular ejection time ratio was found over the upper limit of normal in five patients. For both mitral and tricuspid valves, the mean ratios of peak velocity during passive filling (E) phase of diastole to peak velocity during atrial contraction (A) phase were found significantly lower than in the control group (p < 0.05). Early diastolic peak velocity was similar to that in the control group but late atrial peak velocity was higher in the patient group (p < 0.05). Isovolumic relaxation time was found the same as in the control group. We conclude that cardiac changes in diastolic and systolic functions begin at very young ages in cystic fibrosis patients.